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Neuromyelitis optica spectrum disorders (NMOSD) are central nervous system inflammatory disorders. Recently, autoimmunity against aquaporin-4 (AQP4) water channel is identified to be the underlying immunopathogenetic mechanism of the majority of NMOSD patients. This is evidenced by the detection of IgG autoantibodies against aquaporin-4 (AQP4-IgG) in the serum of ~75%-80% of typical neuromyelitis optica patients. Detection of AQP4-IgG is highly specific for NMOSD, facilitates diagnosis of NMOSD and their distinction from classical multiple sclerosis. Besides its diagnostic value, AQP4-IgG is likely directly pathogenic in NMOSD. This review focuses on the immunopathological effects of AQP4-IgG in NMOSD.

Immunopathological Effects of Aquaporin-4 Ig G in Neuromyelitis Optica Spectrum Disorders